Sprengel’s deformity, also known as high scapula or congenital high scapula is a rare condition where there is an abnormality, where one of the shoulder blades of an individual is higher than the other. This is often caused due to lack of development in the fetus, which results in the condition. It is often not noticed early in an individual. This often restricts movement in the shoulder, in the neck, and sometimes in the upper back of the person. There are also possibilities where the person can develop lumps in the shoulder region, the shoulder blades can also be irregularly shaped. Read on to understand more about the deformity, its signs, symptoms, and possible treatment plans.

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Sprengel's Shoulder: Description

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As mentioned earlier, it is a deformity in the shoulder and neck region, where one of the shoulder blades are higher than the other. This can be visible through a visual examination and on x-rays and other forms of the scan. The person has a hunch, depending on the intensity of the deformity. Usually, the shoulder looks slanted and can have an impact on the person's ability to move. It can have restricted movement on the neck and shoulders, and sometimes can extend to discomfort in using the arms. In most cases, there is little to no pain in the region. The degree of severity varies from individual to individual.

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Classification of Sprengel's Shoulder

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The cavendish classification of Sprengel’s shoulder helps understand the intensity and severity of the deformity. It is classified into four levels, ranging from very mild to severe. Here is the description that allows you to understand it better.

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Very Mild:

 This is the least severe form of deformity, where the shoulder blades are almost at the same level. It cannot be seen when the person is dressed, and there is little to no impact on the movement of the person.

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Mild:

 The shoulder levels are still almost level, but a small lump can be visible when the person is dressed. This also doesn't cause any physical restrictions in movement to a large extent. Minor discomfort may exist.

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Moderate:

 This is when the shoulder blades on one side are elevated at a rate of 2 to 5 centimeters. This stage is easily visible. This can have an impact on how the person functions, and can have an impact on their perception of self due to various reasons.

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Severe:

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This stage is when the shoulder blades are quite high compared to the other and can be closer to the occipital region (near the neck). This can result in restricted movement of the neck, head, shoulder, and even arms. It is visible even when someone is dressed.

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Sprengel's Shoulder Causes

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The exact causes of Sprengel’s shoulder are not known. However, studies have shown that it is a deformity that occurs in the fetal development stage. It is said to occur when the shoulder blades fail to move towards the spine from the neck region during the third month of pregnancy. This results in the shoulder blades staying closer to the neck region and results in deformity. In some cases, a set of abnormal connections made of fibrous bands of tissue can occur in the displaced area of the joints causing an impact on the mobility of the shoulder. In very rare cases, it can occur in people who have had a history of Sprengel’s shoulder cases.

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Sprengel's Shoulder Symptoms

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One of the most common symptoms of deformity is the elevation of one side of the shoulder blades. This is a visible symptom that can also help ascertain the severity of the deformity. Other symptoms are a lump in the spine and shoulder region, and restricted movement of the neck, shoulder, and arms. Often Sprengel’s shoulder is painless. But it can result in discomfort in the neck, head, and shoulder, depending on the severity and the environment the person is from.

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It can be noticed in children and adults, depending on when it is discovered in the person. It is also associated with other ailments such as Scoliosis, Klippel- Feil syndrome, spina bifida, and other spine-related disorders

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Sprengel's Shoulder Diagnosis

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The deformity can be diagnosed in several ways. To begin with, one can look for physical markers such as elevated shoulders, and restricted mobility in the neck. Some of the other forms of diagnosis are through X-rays, where the bones and cartilages area of abnormality can be detected.

 CT or Computed tomography scans can be conducted to identify related ailments such as scoliosis and cervical abnormalities.

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MRI scans can be performed to understand the intensity of the deformity and identify the cartilages and muscular weakness in the shoulder region.

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These are some of the most common ways of Sprengel’s Shoulder Diagnosis.

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Sprengel's Shoulder Treatment

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There are several treatment plans available to treat Sprengel’s shoulder. If it is in young children and the severity is mild to very rare, simple mobility exercises such as swimming, running and other forms of training can help improve the movement of the shoulders. In most cases, surgical intervention can help treat the ailment at various levels. Some of the common treatment plans are as follows.

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Woodward's procedure involves the detachment and reattachment of medial parascapular muscles, which allow the shoulder to move more freely and rotate.

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The Green Procedure involves the extraperiosteal detachment of paraspinal muscles at the scapular insertion and reinsertion after inferior movement of the scapula with traction cables.

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These treatment plans can help improve abduction by 40 to 60 degrees.

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Physiotherapy for Sprengel's Shoulder

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Physiotherapy can be an effective way of increasing mobility in the shoulders of a person with Sprengel’s Shoulder. Sprengel Shoulder physiotherapy can help assist people prior to surgery or can be a good rehabilitation process post-surgery. Here is a quick guide that can help.

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Transcutaneous electrical stimulation can help reduce the pain caused after stretching exercises.

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Strengthening exercises post-surgery can help in increasing the muscle strength of the region and also help the shoulder increase flexibility. Exercises such as shoulder shrugs, rotations, push-ups, and bridging can help in the healing process, all with the consultation of a trained professional.

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Exercises for the Upper trapezius muscle, where the person sits on a chair with their hand under their buttocks and performs neck stretches and flexions with the help of a physiotherapist can improve movement in the shoulder regions.

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Exercise for the Levator scapulae such as performing neck flexions while lying down on your back and using your hands to move the head improves mobility in the region.

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Crossbody stretching is where the person uses their arms to stretch out the other at 90 degrees, and repeats can help reduce pain and stiffness in the region.

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Things to Remember for Sprengel's Shoulder

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• It is a deformity in the shoulder due to a lack of development in the fetal stage.
• It often involves the shoulder blades being higher than the other
• It can result in discomfort and reduction in mobility of the shoulder and neck
• It can be treated with physiotherapy and surgical interventions
• Exercise and flexibility are important to improve living with the condition.

Frequently Asked Questions

What causes Sprengel's deformity?

Research has shown that Sprengel’s deformity can be caused by problems at the fetal developmental stage. During the third month of pregnancy, the shoulder blades fail to move down to their normal position leading to a deformed shoulder and neck. However, the underlying cause of this condition is unknown. 

What is Sprengel's shoulder?

Sprengel’s deformity is a rare congenital condition in which the shoulder blade on one side of the body is too high. The affected and underdeveloped shoulder is also abnormally connected to the spine leading to limited movement. 

How is Sprengel's deformity treated?

There are several treatment options for Sprengel's deformity including simple mobility exercises (swimming, running, etc). In severe cases, surgical interventions are recommended. Some well-known procedures include Woodward’s procedure and the Green Procedure.

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